American Journal of Clinical Dermatology

, Volume 10, Issue 5, pp 331–335 | Cite as

Generalized Sweet Syndrome Lesions Associated with Behçet Disease

A True Association or Simply Co-Morbidity?
  • Serap Köran Karadoğan
  • Emel Bülbül Başkan
  • Gülçin Alkan
  • Hayriye Saricaoğlu
  • Şükran Tunali
Case Report


Sweet syndrome or acute febrile neutrophilic dermatosis is a condition characterized by painful erythematous papules, nodules, and plaques, which demonstrate mature neutrophilic infiltration of the upper dermis histopathologically, and is usually associated with systemic symptoms such as fever and neutrophilia. Generalized Sweet syndrome lesions are usually noted in association with malignancies. Sweet syndrome lesions are rarely seen in patients with Behçet disease and, if present, are usually fewer in number.

In this report, we present two female patients who developed generalized Sweet syndrome lesions during follow-up after the diagnosis of Behçet disease. The clinical and histopathologic findings in our patients together with the data in the literature suggest that generalized Sweet syndrome lesions in a patient with clinically inactive Behçet disease should remind the clinician that a strict differential diagnosis needs to be made between Sweet syndrome associated with Behçet disease and the rare cutaneous manifestation of Behçet disease that resembles the lesions of Sweet syndrome.


Human Leukocyte Antigen Elevated Erythrocyte Sedimentation Rate Erythema Nodosum Leukocytoclastic Vasculitis Erythematous Papule 
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No sources of funding were used to assist in the preparation of this case report. The authors have no conflicts of interest that are directly relevant to the content of this case report.


  1. 1.
    Cohen PR, Kurzrock R. Sweet’s syndrome revisited: a review of disease concepts. Int J Dermatol 2003; 42: 761–78PubMedCrossRefGoogle Scholar
  2. 2.
    Moschella SL. Neutrophilic dermatoses. In: Horn TD, Mascaro JM, Mancini AJ, et al., editors. Dermatology. Edinburgh: Mosby Inc., 2003: 411–23Google Scholar
  3. 3.
    Erbagğci Z, Kirtak K, Özgöztaşi O, et al. Sweet’s syndrome (acute febrile neutrophilic dermatosis). T Clin Med Sci Dermatol 1997; 7: 127–30Google Scholar
  4. 4.
    Honigsmann H, Wolff K. Acute febrile neutrophilic dermatosis (Sweet’s syndrome). In: Fitzpatrick TB, Ersen AZ, Wolff K, et al., editors. Dermatology in general medicine. New York: McGraw Hill, 2003: 949–55Google Scholar
  5. 5.
    Önder M, Gürer MA. The multiple faces of Behçet’s disease and its aetiological factors. J Eur Acad Dermatol Venereol 2001; 15: 126–36PubMedCrossRefGoogle Scholar
  6. 6.
    International Study Group of Behçet’s disease. Criteria for diagnosis of Behçet’s disease. Lancet 1990; 335: 1078–80Google Scholar
  7. 7.
    Chen KR, Kawara Y, Miyakawa S, et al. Cutaneous vasculitis in Behçet’s disease: a clinical and histopathological study of 20 patients. J Am Acad Dermatol 1997; 36: 689–96PubMedCrossRefGoogle Scholar
  8. 8.
    Oğuz O, Serdaroğlu S, Tüzün Y, et al. Acute febrile neutrophilic dermatosis (Sweet’s syndrome) associated with Behçet’s disease. Int J Dermatol 1992; 31: 645–6PubMedCrossRefGoogle Scholar
  9. 9.
    Femiano F, Gombos F, Scully C. Sweet’s syndrome: recurrent oral ulceration, pyrexia, thrombophlebitis and cutaneous lesions. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003; 35: 324–7Google Scholar
  10. 10.
    Hisanaga K, Hosokawa M, Nabuyuki S, et al. “Neuro-Sweet disease”: benign recurrent encephalitis with neutrophilic dermatosis. Arch Neurol 1999; 56: 1010–3PubMedCrossRefGoogle Scholar
  11. 11.
    Lee M-S, Barnetton RS. Sweet’s syndrome associated with Behçet’s disease. Australas J Dermatol 1996; 37: 99–101PubMedCrossRefGoogle Scholar
  12. 12.
    Hisanaga K. Neuro-neutrophilic diseases: neuro-Behçet disease and neurosweet disease. Intern Med 2007: 46 (4): 153–4PubMedCrossRefGoogle Scholar
  13. 13.
    Parlak AH, Kavak A, Alper M, et al. Is a case of Sweet’s syndrome associated with Behçet’s disease? [in Turkish]. TÜRKDERM 2003; 37: 54–7Google Scholar
  14. 14.
    Mizoguchi M, Chikacane K, Goh K, et al. Acute febrile nutrophilic dermatosis (Sweet’s syndrome) in Behçet’s disease. Br J Dermatol 1987; 116: 727–34PubMedCrossRefGoogle Scholar
  15. 15.
    Brady RC, Morris J, Connelly BL, et al. Sweet’s syndrome as an initial manifestation of pediatric human immunodeficiency virus infection. Pediatrics 1999; 104: 1142–4PubMedCrossRefGoogle Scholar
  16. 16.
    Kawakami T, Ohashi S, Kawa Y, et al. Elevated serum granulocyte colonystimulating factor levels in patients with active phase of sweet syndrome and patients with active Behçet disease: implication in neutrophil apoptosis dysfunction. Arch Dermatol 2004; 140: 570–4PubMedCrossRefGoogle Scholar

Copyright information

© Adis Data Information BV 2009

Authors and Affiliations

  • Serap Köran Karadoğan
    • 1
  • Emel Bülbül Başkan
    • 2
  • Gülçin Alkan
    • 2
  • Hayriye Saricaoğlu
    • 2
  • Şükran Tunali
    • 2
  1. 1.Eşrefpaşa Hospital, Department of DermatologyBalcova/İzmir-TürkiyeTurkey
  2. 2.Uludag University Medical Faculty, Department of DermatologyBursaTurkey

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